Wednesday, February 28, 2018

USA news on Youtube Feb 28 2018

Tintype is a photograph, an early type of photography from the 19th century,

that's exposed onto a piece of metal.

We know this soldier's name because it was in the file of a Willis Calhoun of the

67th United States Colored Troops, 67th USCT.

Most of the Civil War pension files are here at the National Archives in Washington, DC.

People come in everyday to review Civil War soldiers' pension files and service records,

as well.

When researchers at the National Archives come across tintypes mixed in with paper records,

they alert Conservation and we do a quick condition check to make sure that the photograph

is stable.

It's then removed from the holdings, scanned by our imaging department, and then

we store it separately in a vault.

This tintype is in very good condition.

You can see some of the emulsion has been disrupted and that the piece of metal

is not entirely flat.

It was really powerful to see the original photo of a person who served in the

Civil War, who fought to end slavery, who fought to preserve the Union.

So it was really powerful to connect to this person who lived long ago, died long ago,

and to see that a real person existed that's in this photo.

For more infomation >> Rare Civil War Tintype Photo Discovered at National Archives - Duration: 1:30.

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Nauert: We will hold Russia to account for bad activities - Duration: 5:42.

For more infomation >> Nauert: We will hold Russia to account for bad activities - Duration: 5:42.

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New satellite photos show Iran establishing base in Syria - Duration: 1:34.

For more infomation >> New satellite photos show Iran establishing base in Syria - Duration: 1:34.

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Hier lagert Russland seine riesigen Goldreserven! - Duration: 0:45.

For more infomation >> Hier lagert Russland seine riesigen Goldreserven! - Duration: 0:45.

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Harrah's Las Vegas Hotel and Casino 2018 Hotel Tour - Duration: 11:16.

For more infomation >> Harrah's Las Vegas Hotel and Casino 2018 Hotel Tour - Duration: 11:16.

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Celebration of a new era - Duration: 44:32.

It was around ten years ago when for the first time I heard of new medicines that were developed...

that were truly different than any other medicine before for CF.

Back then I was still doing relatively really well, my health was really good and back then...

it was not more than just hope that such medicines would come to me or any other CF patients.

Five years later, the first of such medicine was approved for a very small group of patients...

and it was a wonder drug. A drug that changed the life of patients from one second to the other.

In the same year in 2012, I started a new antibiotic.

An antibiotic which was the same which I always used for years but there was one difference.

The difference was that it went from 20 minutes treatment time to just 2 minutes of treatment time.

Simply because the nebulizer changed to a powder form. For me that was an eye opening to research...

more in new technology because I knew from then on I could expect some great things coming up.

In the same year also a new biotechnology company was started - ProQR Therapeutics - where...

I started working a year later. Since then I am in the middle of the heart of CF research.

And now, 5 years later...I am standing here in front of you, you came with a big group of people...

Thank you very much.

Standing in front of you and I can say that I started a new era of medicines...and this is just the beginning.

I would like to welcome you all for tonight. Thank you very much.

It's a very special occasion for me.

You came here from many different groups.

People from all over from England, Italy, here in the Netherlands, family and online.

I would like to give a special welcome to the people who are watching online.

I think it's a unique moment to cherish right now, it's all about now.

And therefore I want to make sure that as many people at home can follow along and celebrating this together.

I would like to welcome my Oma's.

Oma, geweldig dat u er bent. Ik weet ook dat de andere oma nu ook mee kijkt.

Ik denk ook aan mijn opa's. Ik weet zeker dat zij dit ook geweldig hadden gevonden.

Last but not least, I would like to welcome those came from abroad.

They took a plane today coming specially for me. Laura, Hanna, Alexander. Thank you for very much.

I appreciate it a lot.

Tonight I would like to give you an overview of me and CF.

How CF has treated my life so far and also explain what medicines are coming up in the...

upcoming years. And those are a lot.

I also of course I will share my experiences on Orkambi.

Please remember that Orkambi is the occasion I used to celebrate this and not the reason...

why I am celebrating tonight. Because this celebration goes much broader than just starting a new medicine.

I prepared a presentation for now. I am going to speak but feel free to listen to me or watch the screen.

Cystic Fibrosis.

Just a little overview what is Cystic Fibrosis.

It's a genetic disease.

It is one of the most common orphan diseases out there.

Around 70,000 patients world wide have it.

The most orphan disease.

In the Netherlands there are around 1,500 patients.

In the United States around 33,000.

Just to give you a comparison.

It's a multi organ disease.

It affects many organs of you.

Primarily the lungs but also the pancreas, sinuses, liver and bones.

Simply because thick mucous present everywhere downgrades your body.

There are many mutations that cause the exact same disease but the most common one, 50%

of the patients have it worldwide including myself.

Today median survival age around 40.

When I was born in 1990, my doctors told my parents that I would not get any older than

18 and now I am standing here in front of you, 27 years old and doing great as ever

and sill going strong.

But I am lucky compared to other patients who are in a much worse situation and also

compared to my grand nephew, Marco, who is 2 years older than me, and had double lung

transplant 3 years ago.

So this gives a comparison how bad the situation can be.

Now going a little bit technical just to give you an idea what cystic fibrosis is and that

gives a better understanding of presentation later.

It's all about missing a certain protein.

The CFTR protein.

The Cystic Fibrosis Trans-membrane receptor.

This protein is present in any organ that comes in touch with air.

So also your skin.

In a normal healthy person there is a normal flow of water and sodium and chloride.

But someone with CF, there is a chloride channel missing as a result you get a thick layer

of mucous simply because there is a dehydration going on and as a result the mucous layer

isn't able to flow.

We all have mucous but for me it is just thicker than for others.

We have mucous to fight all the particles which we breathe in every single second to

fight any bacteria or virusses.

But for me bad particles is a catalyser to get an infection.

So.

A little bit more about me and CF.

I was diagnosed when I was 3 months after birth.

Of course I don't remember it all but for my parents and family it was a big, what should

I say, shock.

But my parents were great and made sure they took really good care of me, making sure that

I would have a normal youth as much as possible.

And so I did.

I had an extreme healthy childhood.

Absolutely extremely.

I barely noticed anything until I was around 14/15.

I remember in France, I remember a few things of CF.

Not much.

Simply because I didn't get in touch a lot with it.

I remember I had to take my nebulization machine every day.

It was a huge machine.

I remember I had to go to the hospital but the results were the same all the time so

I could move on.

And I remember my mom or my dad treating me every morning and evening, batting on my lungs

to get the thick mucous out of lungs.

Physiotherapy.

But maybe what I remember most is actually the experience I have had in Paris.

One time we went to Paris, to Disney land Paris, and I had to do my nebulization treatment

and the machine exploded.

Literally.

So the result was that the whole hotel had a power cut.

That is what I remember of CF in France.

It was good fun.

When I lived in England similar things happened.

Nothing much.

I every now and then I went to the hospital and I started taking oral antibiotics for

the first time on a regular basis just to make sure I wouldn't get sick.

For those of you that are here, Dries and Merel, you probably havn't seen much of me

and CF.

At school I was doing really fine, like all the others.

But Dries, probably does remember I had to the nebulization treatment every now and then

and that I coughed every now and then.

Nothing much more.

Then I moved to Italy.

Things were going the exact same way.

There was one difference though.

I went to an Italian hospital.

And Italian hospital is an experience on its own.

Let alone it was a hospital of the Vatican city.

Meaning it was a Catholic hospital.

And firs hospitalisation was in 2004.

It was not so much that I was very sick but more a preventive cure to prevent worse.

And it was one to never forget.

Seriously.

Not so much because it was the first time.

Not so much because it was a little uncertain what was going to happen next.

More because of the second day I was in the hospital.

There was an issue at the department next to me.

There was a virus infection going on so the chapel which was next door was not going to

be used.

So they decided without my permission but just by doing it, to do the Sunday mass in

my room.

So the nurse came to our room and said 'listen you have the biggest room in the near area

so we will do the sunday mass in your room, together with your roommate.'

So my dad and I together with 15-20 other people we had an entire mass.

Welcome to Italy.

Scusa Laura, ma..e cosi.

Laura: "It's the Vatican, it's not Italy.'

-Aaaaaa, vero.

Bava brava, hai capito bene.

I had my first real infection in 2006.

There I had a severe lung infection.

Again it was in an Italian hospital.

I moved to a new department, new building so that on its own was an experience.

The room wasn't finished yet and there was big chaos.

Like always.

But it was a serious infection.

Since then I slowly slowly developed more and more mucous.

But, it was still relative.

For those of you, the Italians that are watching, at school I every now and then went to the

hospital a few weeks.

I had get some antibiotics.

I coughed a bit more.

But in the end my IB diploma and American High school diploma like all the others without

any delay so that was a big achievement as well.

Then I moved to the Netherlands and things went smooth.

They continued to go smooth.

First few years were very stable.

Much more stable than in Italy.

Which was surprising actually.

In May of 2012 I had an infection.

A lung infection where I went to the hospital.

That month, it was May of 2012 was an eye opener for me.

In that month I started a new antibiotic which was a powder form.

An inhalation form.

Treatment time went from 30 minutes to 2 minutes a day.

And so that made me interested to read about the technology of CF.

Just a year later I went to work at ProQR.

But before that, I had a dream to go abroad to the United States for an exchange.

That was first delayed but at the end I was able to go without any problems and I had

an amazing time over there.

There in the US I learned more about CF.

I got into touch with the CF Foundation and I came to realise that here in The Netherlands

we have a really good healthcare system.

All the medicines, or most of them, are covered and there in the US, CF patients fighting

with their insurance companies to get their medicines or sometimes they don't get access

at all to the simple medicines out there.

So that was a realisation that here in the Netherlands we have it really really good.

When I came back I all of a sudden got this call from my recruitment agency because there

was this company in Leiden which was looking for someone with Cystic Fibrosis.

Are you interested to maybe have a chat?

And so I did and within no time I was hired there and the rest was history because I still

work there one day a week and it is a unique experience to be so close to the research

of CF drugs.

These were the medicines which I took to the States for half a year.

It was actually a lot of administration needed.

Also because a Dutch guy, a Dutch young guy travelling to the United States with white

powders and liquids in your hand luggage, you simply ask for problems.

But I was well prepared it worked out well.

Until then I was doing relatively doing well.

A lot of people here are from badminton.

They know me from badminton.

And they have seen that I can play badminton like all the others without any problems.

Sometimes I coughed a bit more than the other days.

But in the end I could play competition, I could

play the games like any other.

I also had a lot of fun during the third half.

Drinks afterwards and that went without any issues at all.

And then also followed my master's degree.

My first year of my masters degree, also that went really well without any major issues.

But since then things started to change a little bit.

I got the chronic pseudomonas infection.

It's a bacteria, a severe bacteria.

It's unique I got it so late.

Usually you get it much younger and as a result you get much more sick.

But that for me was a moment where I had to make more antibiotics due to infections with

more mucous.

Last year was my worse year ever.

I had a severe pneumonia infection in the beginning of the year.

And in the second half I had another hospitalisation unfortunately.

Simply because of the pseudomonas coming back.

So in the total I had 12 cf-related hospitalisations in my life.

That may sound a lot but that still is relatively low.

Just one more thing about me'nd cf.

To compare my lung function.

Because I hate comparing me to other cf patients.

CF is a very complex unique disease.

It is different for everyone else.

But clinically you have to put measurements around it and that is the lung function.

This graph shows median lung function over the years.

And what I tell you right now, is that if you are 27 years old, the median lung function

is around 65%.

This means that 50% of the patients have a lung function of around 65% or lower.

Which means that they have lost 40 to 45% of their lung function.

This slowly means you get quicker out of breath.

Infections.

Not being able to do sports as much and all that.

Just to compare, in November of last year, I was off this chart.

My lung function was 110% which is truly unique.

There are not many people.

There are not many people that can achieve that.

My doctor reminds me every time, also to myself, I am very happy to have such a lung function.

Even though I have a lot of other issues and complaints, this is still something to be

very proud of.

I want to give a little overview of the medicines that exist so far for CF because this is very

important to understand what the current medicines which just came out are different than any

other medicine before.

What I just explained in the image is that people with CF have a thicker mucous and as

a result you have more mucous in your lungs.

You get lung obstruction.

Your lungs are filled up with mucous instead of air, therefore you can breathe less.

You are quicker out of breath and you have less energy.

This is the perfect environment for bacteria to grow in for infection.

Bacteria love thick mucous.

It is relatively wet and warm so you can get an infection.

With in infection you get tissue damage.

Your tissues become red and thicker and so your lung airways become smaller and you get

out of breath.

Of course your human body won't be your human body if it wouldn't react so as a result your

body reacts in trying to fight this infection.

How does it do that?

By creating mucous.

By killing the bacteria it creates mucous so that they can get out of your lungs.

But people with CF already have thick mucous due to the defect.

So as a result you end up in this vicious circle where you go from thick mucous to more

thick mucous to more thick mucous.

And over time this results in respiratory failure, lung damage, lung failure and so

forth.

This is a complex process simplified.

It's a short term process but it is also a long term process.

In the short term you get an infection and in the long term you get lung decline.

To compare, healthy people who get a lung infection or a flu or a cold go through the

same circle, exact same thing.

But the only difference is that they get out of it, while for CF patients it's a catalyst

to get more sick.

So for each of these steps there are many different types of medicines.

I am not going over all of them.

There mucous thinners, mucous enzymes that try to cut the mucous, mucous clearance techniques.

So with physiotherapy, chest physiotherapy, physiotherapist, sports, to try to remove

the mucous as much as possible from your body.

Antibiotics which try to treat the infection and the anti inflammation medicines reduce

the inflammations.

Over time if these medicines don't work anymore you slowly move towards a lung transplant.

But that's a long process and much more complex.

So on a daily basis, I take around 20-25 medicines a day, if not more.

Treatments in the morning, afternoon and evening.

That's been my whole life and still will be for the upcoming years.

Okay, but now comes the crux of this whole story.

These medicines treat the symptoms of CF.

They try to reduce the mucous.

They try to remove the mucous and try to remove the infection so that less thick mucous is

present in your body.

But in past 5 years, things changed so much.

Because medicines are developed that treat the underlying cause of the disease, making

sure no thick mucous will be developed, so that this defect repaired and as a result

no more thick mucous is developed at all.

Over time you hope to achieve a result where the vicious circle is stopped or reduced so

that you take less additional medicines.

5 years ago, Kalydeco was approved for a very small group of patients and Orkambi was approved

3 years later in the US.

Orkambi is also the medicine which I start taking in November.

These medicines have changed the world for many CF patients already.

You can't imagine.

I can't tell on behalf of them.

They have to tell that themselves but if you hear the stories, if you read the stories

and much of an impact these medicines are.

It's truly amazing.

From not able to walk, not able to do anything to just having a life again.

Able to work, thinking of getting a family.

Thinking of doing fun tings because you can breathe again.

We breathe every single second, every single day.

If that's an issue than that has a big impact on your life.

And if that simple thing can change the life, than that's truly amazing and a miracle.

Kalydeco is truly a wonder drug.

It's absolutely a wonder drug.

Surreal what that medicine has done.

Scientists and everyone is amazed about this medicine.

Orkambi is less.

It's more suspicious and there are more side effects but also that medicine has changed

the life of many.

Yesterday, or actually last week, I talked to patient who was in a wheelchair.

Was not able to breath or walk anymore to ice skating 100 km in just one year.

That is how impactful this medicine can be.

Okay, so a little controversial here.

Costs of medicines.

I think that is very important to mention.

How expensive are these medicines?

Unless if I told you any of you already in person, does anyone have any idea how much

the costs are on a yearly basis for the regular treatments which I just explained to you right

now.

Any idea?

A small house?

In the Netherlands btw.

So if you just look at the medicines.

If you just look at the medicines we are talking about €50,000 a year.

If you include the regular hospital check-ups which are 4-5 a year you can add another €5,500

- €6000.

If you have an hospitalisation and you count 2 per year.

It's another €20,000.

So all in all, right now I am costing...well now a bit more....but before I started Orkambi,

I costed €80,000 including all these medicines.

Now, to put it in perspective.

Orkambi costs €180,000 a year.

More than double than what I already cost right so far.

So that put this whole costs in a complete different range.

Whether you agree with it or not, I am not going to discuss that tonight, but it does

put things in perspective how these medicines are.

And that just in the Netherlands.

In the US, I can tell you the costs are double.

At least, yes.

This excludes any vitamins, any additional medications, physiotherapy, all the nebulization

stuff and that is still quite a lot.

By the way, if anyone is interested to look up their own costs in the Netherlands go to

medicijnkosten.nl.

There you can look up any medicines.

By law they are required to say how much the costs are.

That is why you can see the exact prices.

That being said, Orkambi I have mentioned the market price not the real price because

there is a financial agreement which is secret, but the market value is still €180,000.

Now, to the interesting part.

CF research.

The pipeline of CF research.

Today there is so much CF research going on.

There are around 20 known modifying disease researches going on worldwide that threat

the underlying cause of the disease.

That's a lot.

Thinking it's an orphan disease, it is a lot of research.

It's all about repairing CFTR, that protein.

It's all about that.

Repairing that protein can be done in many stages of the CFTR development proces.

DNA, RNA and the protein.

You are standing in the kitchen, DNA are your ingredients, RNA is the recipe, and the protein

is the cooking part.

The meal you are making.

At all these levels, medicines are being developed to try the meal which you are making.

This amount of research has increased exponentially in the past 5 years on all these stages.

So I work at ProQR Therapeutics, there they develop RNA therapy trying to fix CFTR on

a RNA level.

But the most common, biggest group of companies are working trying to fix when the meal is

being made by the cook.

Trying to add some molecules in the last minute so that the CFTR protein is repaired.

That is a big group of research and very intresting to follow.

Vertex, is the company which developed Orkambi has already their follow-up medicine ready.

In fact, their follow up medicine of Orkambi which I am eligible for, will be approved

most likely on February 28th of this year.

In the second half of 2018 it will be approved in Europe and I expect it to be available

in The Netherlands around 1,5-2 years.

If that is not enough, they are already with a third generation medicine which is much

better.

The follow up medicine of Orkambi, the 2nd generation is more treating the side effects.

It is not necessarily better but has less side effects.

While the 3rd generation is much better.

The most interesting about this medicine is that it treats much more patients than just

the 50%.

Therefore over 90% of the patients are expected to have access to this medicine.

And then there are other companies which are doing similar things, trying different techniques

to treat the underlying cause of the disease.

Last year I was actually for a trial from Galapagos to participate.

I declined because of practical reasons but I definitely looking forward to this year

where new trials are coming up and I am sure I might be eligible for it, even though I

am on Orkambi right now.

And that is just the research trying to treat the underlying cause.

There are over 20 other clinical trials at the moment, CF-related, and are different

and try to reduce the infections, try to reduce the mucous and other type of research.

And also that is promising.

This is very technical.

But we all know that antibiotics is bad for us.

We all know that antibiotics creates resistance.

And so for CF patients it is crucial that new medicines are developed that fight the

symptoms so that infections can be treated better.

Most CF patients will become resistant to all antibiotics and then you just decline

very fast.

So in this area of research, also for me, it is very interesting to follow what Is going

to happen.

Then there is a very special category of medicines.

They are the personalised medicines.

Personalised medicines is a new technique where they try to look at each individual

patient to see if a certain drug or research drug will be working on you personally.

The Netherlands is actually leading in this.

UMC Utrecht, just right across my window where I live, they are developping new technology

where they take a sample of your gut and with that sample they are able to test if medicines

and predict if the medicines will react or not on you.

You can imagine that is very interesting because insurance companies are very interested to

see if a medicine will work or not and predict beforehand if such a medicine which firstly

was not researched for you can be applied to you.

And it is unique that in The Netherlands 3 Dutch insurance companies have invested in

this project to get this moving forward because the patients who suffer the most right now

are the patients that do not get access to such new medicines because they fall out of

these mutations.

They have a very rare mutation resulting in no hope that such medicines would ever come

to them.

But this is a solution for them because with this they might get medicines for them personally

to get it to work.

Every CF patient deserves disease modifying medicines like I do.

Everyone of them.

And that's why I decided....That's why I decided that the money which you donated to me tonight,

which I would like to thank you very much for, I am going to give it to a cf targeted

research project that does more research in trying to understand better how the CFTR works

in those very rare mutations so that get more hope for such medicines.

I think that's very important.

All CF patients diverse the exact same medicines so also those with a very rare mutation.

So, what about me and Orkambi?

Orkambi, the medicine which I started taking 2 months ago.

You have all read about it in my blog or you've heard it in person but I hope by now it's

clear that Orkambi is not the reason I'm celebrating here.

It's just the occasion.

Orkambi is just an additional reason why I am celebrating this with you all.

See, Orkambi and me is a personal special story on its own.

Yes it's a medicine which treats the underlying cause.

Yes, its's finally available in The Netherlands but for me it goes much further beyond than

November 1st when it was approved.

Because I work at ProQR Therapeutics I have a very unique insight on the development of

this medicine.

I know all the in's and out's of this medicine.

The pro's, the con's.

Side effects.

Should it be approved?

Should it be reimbursed?

Yes or no and so forth.

so in the office, during working hours and afterwards I had many technical discussions

and many other talks and things about this medicine.

Whether this will work but also from a business perspective how will it affect our research?

How will it affect ProQR development process?

So from a personal point of view, I was very excited that this medicine was finally approved

4 years ago but from a business and competitive perspective it was very interesting to this

would influence us.

Well , it took a long time before Orkambi was finally approved in the Netherlands.

It was approved in the United States in 2015, July 2015.

I remember the moment.

I was actually with my GiMA students, master students, we were sitting at the sea, sunset,

last night, it was a good night, right?

But you might not know that at that moment they approved the medicine.

For me that was also a wake up call 'wow, now it's truly very close.

I just finished my first year of my masters degree and there is a medicine nearly in my

hands.

Well I thought back then.

But the US patients it was.

That was truly a unique moment.

It took unfortunately another 2.5 years to approve here in The Netherlands.

Negotiations took way too long, but once it was approved, it went really fast.

It was approved on October 24th 2017 and just a week later it was available.

That's how fast it can go sometimes.

So I remember it was my last day of intravenous antibiotic cure I had back then in October.

it was my last day of intravenous antibiotics and on the same day Orkambi was approved in

the Netherlands just a week later.

It was such a double feeling, because I wasn't feeling well that day but I knew something

might be coming up which would change me once and forever.

And I never thought to start so quickly.

Because on October 31st, six days later I got a call from my doctor saying 'Listen Jelle,

Orkambi is approved, do you want to start?'

Yes I do.

-'ok,, come to the hospital on November 2nd.

We will do a baseline test and from then on you can start taking the medicine.'

My parents and I were so amazed how fast it went.

I couldn't believe it.

So I went to the hospital on November 2nd and that was a special day as well.

That day many CF patients came to the hospital and you could see from the employees there,

the doctors, nurses, and also for them it was a special day.

They felt this medicine could change patients a lot and even though they had to work hard

they were all happy and very excited for us.

So even for them it was a very special moment.

On November 6th, I got the medicine in my hands and I remember the truck driver had

a half million worth of Orkambi in his truck.

Even for him, he said, it's a special day for me because I am able to make people happy

and that is what I love this job.

Also for him it was a very special moment he said.

So I started taking Orkambi immediately.

I celebrated it with my parents and sister.

I wanted it to combine it with tonight but it went way too fast otherwise I would have

taken tonight my first dose just to show Orkambi is the not reason why I am celebrating but

just the occasion.

And then, and then.

It only took 3 tablets and 20 hours to feel the most wonderful experience I have ever

had.

3 tablets did the exact same thing as intravenous antibiotics would do in 2-3 weeks.

My thick mucous was gone.

Within 1 day.

It was truly wonderful.

When I woke up on Wednesday, on day 2, I did a deep breath in and I was like ' WOOOOOOW'.

This can't be true right?

It was such a wonderful experience.

And that week my voice changed, my nose changed, it went open.

I had a lot of energy.

It was a lot of euphoria but you could see, you could hear that things went really well.

I knew immediately this medicine is working for me.

For some patients a long time before they see a reaction and some patients they have

to stop quickly but for me knew this is working.

It's truly amazing.

Those experiences continued.

When I took my bike, it was another way of breathing.

When I played badminton it was another way of coughing up mucous at the end of the day.

While I am presenting here, my breathe is so relaxed.

I don't remember it has ever been so relaxed as ever before.

The amount of energy I had in the past 2 months is truly amazing.

I already do a lot on a daily basis.

Masters degree, work, this, that.

I couldn't believe that I could gain so much more energy simply because there is less thick

mucous in my lungs that try to be frighted against.

That on its own is amazing.

2 weeks after I took Orkambi, unfortunately my liver enzymes were too high which I maybe

had to stop.

It is a common side effect.

But luckily they went back down again.

In my 3rd week when it was uncertain if I was able to continue or not, a lot of people

asked me so are you worried about it?

And I said listen, I am experiencing such wonderful thing right now that I am not been

thinking about it too much.

And before I knew it was already over so it did not distract me a lot.

But it is true though, that the liver enzymes is still a thing to look after at and keep

in mind because it still might be a reason why I need to stop in the longer term.

CF is not over.

Definitely not.

But Orkambi did give me many new experiences.

And that is what counts the most.

Because I have to say, I have to be honest.

Unfortunately, I started new antibiotics this week.

It was quite unexpected.

It is quite unexpected but it was needed.

And that shows that CF is definitely not over.

But what I have experienced so far these 2 months is truly amazing and I will cherish

that forever.

And that is what is most important.

Believing in hope, having dreams but if those dreams come closer to you, experience those,

you have to cherish that as much as possible.

And that is the reason why I am with you all, including those that are online, to be part

of this experience.

I hope tonight that I have given you an idea on me and CF, what is coming ahead and what

Orkambi has done to me.

I know some of you are still studying or are working in biotech, pharmaceutical industry

and you have to remember that CF is just the first disease, one of them, but there are

more diseases to come where similar experiences will follow.

CF is just quite unique to be one of the first.

And that also motivates me to keep on studying and learning more about other diseases.

Because I have experienced how truly amazing one medicine can be, and so hope that so many

more patients will follow from now.

Thank you very much for your attention.

Cherishing the moments like today is what I do, that is how I live.

I think it is great that I am able to share this with you around the world with you all

and I truly hope that we can celebrate many moments to come.

Thank you very much.

Cheers.

For more infomation >> Celebration of a new era - Duration: 44:32.

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Styling and Makeup for Bikini Photoshoot | Fashion Photography Tutorial | Part 1 and 2 - Duration: 2:20.

Hi, This is Ryan.I am a photographer specialised in Fashion and Nude.

This is the second video of the Fashion and Nude Photography Tutorial Series.We have done

the first tutorial with this red bikini as styling and we learned about ISO, its relevance

and all.

So, we have model Sneha here.

I wore this Bikini and that colour is very nice.

That's great and I will try this one.

You can try this with this one.

So, as I was saying in first Tutorial.

These kind of Bikinis are like for example, it's a polka dot Bikini.

Either you can wear it without any accessories or any cover-ups or something like that.

But, in this tutorial as a change we will use this along with the bikini and I think

this also a Polka dot and that would be great.

We are doing Makeup for second tutorial.

You can see that she is wearing the polka dot bikini which we selected during the styling

and she is wearing the cover-up also.

And, if you have seen the first tutorial, this is the consecutive.

We are shooting consecutively.

The makeup and everything remains same, she is having the same red lipstick and we will

do some touch up for eyes, maybe blush or something like that.But, the main makeup remains

same.

She is having the red lipstick and red nail paint and pink eye shadow also remains same.

So, we are planning to do at least 3 tutorials today to give you an idea about ISO, Aperture

and Shutter speed.

First tutorial was about ISO.

In this tutorial we will give you an idea about Aperture.

I think this is all you need to know about Makeup.

Now, we will see the lighting.

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